Type IV Ehlers-Danlos syndrome with isolated arterial involvement.
Ehlers-Danlos syndrome is a connective tissue disorder characterized by skin hyperelasticity and friability, hyperextensible small articulations, vascular fragility, and ocular and visceral symptoms. Ten types of Ehlers-Danlos syndrome have been described to date. Type IV is characterized by isolated arterial involvement with the risk of rupture, making diagnosis difficult. A 25-year-old man was operated on for a ruptured left and then right iliac artery and once again for a hepatic artery aneurysm within a nine month period. The diagnosis of Ehlers-Danlos syndrome was based on clinical and histopathologic findings. An asymptomatic iliac artery aneurysm was found in the patient's 23-year-old brother.
Bellenot
F
F
Service de Chirurgie Thoracique et Vasculaire, Centre Hospitalier, Universitaire de Clermont-Ferrand, France.
Boisgard
S
S
Kantelip
B
B
Maillard
P
P
Tissandier
P
P
Ribal
J P
JP
Glanddier
G
G
D000328:Adult; D000783:Aneurysm / Q000150:complications* / Q000175:diagnosis / Q000601:surgery; D004535:Ehlers-Danlos Syndrome / Q000145:classification / Q000150:complications* / Q000175:diagnosis; D006406:Hematoma / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006499:Hepatic Artery; D006801:Humans; D007083:Iliac Artery; D008026:Ligation; D008297:Male; D012187:Retroperitoneal Space; D012422:Rupture, Spontaneous